原发腮腺淋巴上皮癌临床特征与诊断及疗效分析

目的 分析原发腮腺淋巴上皮癌的临床特征、诊断特点及预后。方法 回顾性分析2009-2017年经中国医学科学院肿瘤医院确诊的13例腮腺淋巴上皮癌患者临床资料,中位随访时间38.5个月,所有患者均先行手术+放疗。结果 全组13例患者中男9例,女4例,中位年龄33岁。确诊时局限于腮腺9例、区域淋巴结转移4例,均为Ⅰ b、Ⅱ区。UICC2010分期Ⅰ、Ⅱ、Ⅲ、Ⅳ期患者分别为1、1、6、5例。11例患者手术病理标本EBER检测10例阳性。全组无死亡事件,3年总生存率100%,3年无进展生存率76%,3年局部控制率92%,3年无远处转移率84%。结论 腮腺淋巴上皮癌发病率较低,病变与EBV相关,容易发生颈部淋巴结转移,疗前应除外鼻咽癌转移至腮腺的可能。目前治疗模式是以手术+放疗,总体预后较好,治疗失败主要原因为局部复发和远处转移。

Clinical characteristics,diagnosis and efficacy of primary lymphoepithelial carcinoma of the parotid gland

Objective To analyze the clinical features,diagnosis and prognosis of patients with primary lymphoepithelial carcinoma of the parotid gland. Methods Clinical data of 13 patients diagnosed with lymphoepithelial carcinoma of the parotid gland in our hospital from 2009 to 2017 were retrospectively analyzed. The median follow-up time was 38.5 months. All patients received radiotherapy after operation. Results Of 13 patients,9 cases were male and 4 female. The median age was 33 years. At the initial diagnosis,9 cases had primary lesions limited to the parotid gland,and 4 cases of lymph node metastases located in Ⅰ b and Ⅱ regions of the neck. According to UICC2010 staging,1 case was classified as stage Ⅰ,1 as stage Ⅱ,6 as stage Ⅲ and 5 as stage Ⅳ, respectively. Eleven surgically pathological specimens were tested with EBER in-situ,and 10 cases were positive for EBER. No patient died in the whole group. The 3-year overall survival rate was 100%. The 3-year progression-free survival rate was 76%. The 3-year local control rate was 92%. The 3-year metastasis-free survival rate was 84%. Conclusions The incidence of lymphoepithelial carcinoma of the parotid gland is relatively low. The pathological features are associated with EB virus. It is prone to present with cervical lymph node metastasis. The possibility of lymph node metastasis of nasopharyngeal carcinoma to the parotid gland should be excluded before treatment. At present, surgery combined with postoperative radiotherapy is the main treatment. The overall survival is favorable. Local recurrence and distant metastasis are the main causes of treatment failure.